Integra™ appears to be a suitable coverage for large soft tissue defects in utero. Moreover, a postnatal reverse latissimus dorsi flap appears to markedly strengthen tissue coverage over a spinal cord rescued in utero.
To describe the prenatal course and perinatal outcome, and to define prognostic markers for fetuses with congenital pulmonary airway malformation (CPAM) or bronchopulmonary sequestration (BPS). A retrospective study was performed at the University Hospital Zurich including pregnancies with either fetal CPAM (n = 26) or BPS (n = 11) between 2000 and 2013. Three patients decided for termination of pregnancy. Two intrauterine deaths (CPAM) occurred at 25 weeks. Minimally invasive interventions were performed in 9/37 (24 %) fetuses, post-interventional survival was 8/9 (89 %). Mean gestational age at delivery was 38.1 +/-2.8 and 39.1 +/-2.5 weeks in fetuses with CPAM or BPS, respectively. In fetuses with CPAM the perinatal mortality rate was 4/24 (17 %); the rate of invasive interventions or surgery during the early neonatal period (neonatal morbidity) was 9/22 (41 %). Prenatal diagnosis of hydrothorax and/or increasing cystic volume ratio (CVR) until delivery preceded perinatal death in 3/5 (60 %). Absent mediastinal shift showed a neonatal morbidity rate of 1/8 (13 %) without any perinatal mortality. In fetuses with BPS the perinatal morbidity and mortality were both 1/10 (10 %). Hydrops predicted morbidity and mortality in 100 % of cases. Absent hydrops was followed by uncomplicated perinatal outcome. Fetuses with CPAM or BPS have a good outcome under optimal perinatal care including the possibility to perform minimally invasive prenatal interventions. CPAM without mediastinal shift and BPS without hydrops have an excellent prognosis. Hydrothorax, increasing CVR or hydrops indicates a high risk for perinatal morbidity and mortality.
In the past 40 years, the percentage of twin pregnancies has increased by almost a third as a result of a rise in medically assisted reproduction and delayed childbearing. [1] Of the 1.6 million twin pairs born around the globe every year, %15% are monochorionic (MC), i.e., they share the same placenta. [2] These pregnancies present more frequent complications than dichorionic twins that develop with separate placentas. [3] One of these complications arises from vascular anastomoses that connect the blood circulation systems of both fetuses to the placenta. Twin-to-twin transfusion syndrome (TTTS) affects 10-15% of MC multiple pregnancies and is characterized by a chronic, imbalanced blood flow from the donor to the recipient twin, which results in a disproportionate nutrient supply. [4] If left untreated, the consequences of TTTS are severe, leading to a mid-trimester mortality rate of up to 95%. [5] State-of-the-art treatment of TTTS involves fetoscopic laser coagulation of the placental anastomoses. Under ultrasound guidance, the surgeon identifies a safe entry site in the maternal abdomen from which a fetoscope is inserted through a trocar (typically 2.2-4 mm in diameter; 7-12 French [6] ) into the recipient's amniotic sac. The fetoscope consists of a camera and a working channel to deliver laser light through an optical fiber at the desired location. Before the surgeon ablates the vessels, the vascular architecture is scrutinized and the connecting vessels are identified. Subsequently, all identified anastomoses are coagulated with a neodymium-doped yttrium aluminum garnet (Nd:YAG) or diode laser such that the MC circulation is converted into two independent vascular systems. [7] To ensure no small vessels are missed, the laser is repeatedly fired along a line connecting all the coagulation points from one placental border to the other (known as the Solomon technique). [5,8,9] Mortality rates still range from 20% to 48% after this surgical procedure and significant complications are reported in 6-18% of surviving newborns. [10] Neurological damage to the fetus is also more likely to occur in technically difficult cases. [8] As the procedure is demanding, outcomes are also dependent on the surgeon experience. [7,10] Cases with anterior placentas (i.e., located on the abdominal side of the uterus) constitute a major challenge, even for experienced surgeons. Good access and visualization of anterior placentas are difficult with rigid endoscopes. [11] This can prevent complete coagulation, which,
Background: One of the intraoperative challenges of fetal spina bifida repair is skin closure when there is an extended skin defect. Thus, we examined whether distally pedicled random pattern transposition flaps (TFs) are a valid option to overcome this problem. Subjects and Methods: All patients undergoing in utero repair of spina bifida with application of a TF for back skin closure were analyzed focusing on intraoperative flap characteristics and postoperative flap performance. Results: In 30 (70%) of the 43 fetuses a primary skin closure was achieved, in 5 (12%) a skin substitute was used, and in 8 (18%) a TF was applied. Flap raising and insertion was uneventful and perfusion was sufficient in all 8 fetuses (100%). In 3 fetuses (37%) the donor sites were closed primarily, and in 5 (63%) a skin substitute was used for coverage. At birth, 7 flaps were viable and provided robust skin coverage over the center of the former lesion. Complications included a small skin defect with CSF leakage in 1 patient (13%). Conclusion: During open fetal spina bifida repair, TFs can be safely and efficaciously used to obtain solid and durable skin coverage over lesions too large to allow conventional primary skin closure.
Purpose The aim of this study was to describe the sonographic evolution of fetal head circumference (HC) and width of the posterior horn of the lateral ventricle (Vp) after open fetal myelomeningocele (fMMC) repair and to assess whether pre- or postoperative measurements are helpful to predict the need for shunting during the first year of life.
Patients & Methods All 30 children older than one year by January 2017 who previously had fMMC repair at the Zurich Center for Fetal Diagnosis and Therapy were included. Sonographic evolution of fetal HC and Vp before and after fMMC repair was assessed and compared between the non-shunted (N = 16) and the shunted group (N = 14). ROC curves were generated for the fetal HC Z-score and Vp in order to show their predictive accuracy for the need for shunting until 1 year of age.
Results HC was not an independent factor for predicting shunting. However, the need for shunting was directly dependent on the preoperative Vp as well as the Vp before delivery. A Vp > 10 mm at evaluation for fMMC repair or > 15 mm before delivery identifies 100 % of the infants needing shunt placement at a false-positive rate of 44 % and 25 %, respectively. All fetuses with a Vp > 15 mm at first evaluation received a shunt.
Conclusion Fetuses demonstrating a Vp of > 15 mm before in utero MMC repair are extremely likely to develop hydrocephalus requiring a shunt during the first year of life. This compelling piece of evidence must be appropriately integrated into prenatal counseling.
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